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Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study.

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Systemic sclerosis patients without pulmonary arterial hypertension (PAH) show worsening exercise hemodynamics and reduced exercise capacity over 4 years. The incidence of PAH was 0.75 cases per 100 patient-years.

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Area of Science:

  • Cardiology
  • Rheumatology
  • Pulmonology

Background:

  • Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis.
  • Early detection and monitoring of pulmonary vascular changes are crucial in scleroderma patients.

Purpose of the Study:

  • To prospectively monitor changes in resting and exercise pulmonary hemodynamics in systemic sclerosis patients without initial PAH.
  • To determine the incidence of developing PAH in this cohort over a mean follow-up of 4 years.

Main Methods:

  • Prospective cohort study involving 99 systemic sclerosis patients.
  • Utilized exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up.
  • A subgroup underwent exercise right heart catheter (RHC) investigations for detailed hemodynamic assessment.

Main Results:

  • A significant increase in exercise systolic pulmonary arterial pressure (sPAP50) and a decrease in peak oxygen uptake were observed.
  • In the RHC subgroup, exercise mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance increased significantly.
  • Three patients (3%) developed RHC-confirmed PAH over the follow-up period (incidence: 0.75 cases per 100 patient-years).

Conclusions:

  • Systemic sclerosis patients without baseline PAH experience a gradual deterioration in pulmonary exercise hemodynamics and exercise capacity.
  • These findings suggest subclinical pulmonary vascular disease progression in scleroderma.
  • Regular monitoring for PAH development is warranted in this patient population.